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Intrinsically disordered proteins or regions of proteins lack a well-defined structure, yet they carry out important functions often associated with the regulation of cell cycle and transcription. Due to these central roles in key cellular processes, their mutations are frequently involved in neurodegenerative diseases. These diseases are usually caused by the structural transition of disordered proteins...
Neurodegenerative diseases constitute a set of pathological conditions originating from the slow, irreversible and systemic cell loss within the various regions of the brain and/or the spinal cord. Depending on the affected region, the outcomes of the neurodegeneration are very broad, starting from the problems with movements and ending with dementia. Neurodegenerative diseases are proteinopathies...
Protein aggregation as a result of misfolding is a common theme underlying neurodegenerative diseases. Although a subject of intense research, how misfolded proteins bypass sophisticated protein quality control measures in the cell to be deposited as ubiquitin-enriched inclusion bodies remains poorly understood. Whilst proteasome dysfunction could account for this phenomenon, emerging evidence suggests...
Genetically engineered mouse model studies show that neuronal dysfunction caused by protein aggregation/misfolding are reversible, indicating that injured neurons are alive even under disease states. Protein misfolding/aggregation in axons and distal dominant axonal degeneration are observed in a subgroup of degenerative diseases and in certain experimental conditions. Moreover, therapeutic approaches...
Accumulation of misfolded proteins in the endoplasmic reticulum triggers a cellular stress response called the unfolded protein response (UPR) that protects the cell against the toxic buildup of misfolded proteins. Neurodegenerative disorders like Alzheimer’s disease, Parkinson’s disease, prion disease, Huntington’s disease, frontotemporal dementia, and amyotrophic lateral sclerosis are characterized...
The protein alpha-synuclein is implicated in the etiology of both sporadic and hereditary Parkinson’s disease. Structural studies of both the intrinsically disordered free state of the protein and of more ordered states, adopted when alpha-synuclein self assembles into fibrils or binds to lipid membranes or detergent micelles, have begun to provide insights into factors that likely influence both...
Parkinson’s disease (PD) is a neurodegenerative disorder involving a loss of dopaminergic neurons from the substantia nigra. A characteristic feature of the post-mortem brains of PD patients is the presence in surviving neurons of Lewy bodies, cytosolic inclusions enriched with fibrillar forms of the presynaptic protein α-synuclein. Upon prolonged incubation at physiological temperature, α-synuclein...
α-Synucleinopathies are a group of neurodegenerative disorders characterized by the presence of intracytoplasmic Lewy bodies in Parkinson’s disease and dementia with Lewy bodies as well as glial cytoplasmic inclusions in multiple system atrophy. The main component of these inclusions is aggregated α-synuclein which supports a strong link between α-synuclein and disease pathogenesis. The mechanisms...
There is increasing evidence that unfolded and misfolded proteins initiate a cascade of pathogenic protein-protein interactions that culminate in neuronal dysfunction. This is a multistep process which results in toxic protein aggregates; thus they are potent targets for development of early diagnosis and of drugs to improve therapies of conformational diseases. The hallmark proteins of these diseases...
Neurodegenerative diseases are characterized by death and progressive loss of neurons in distinct areas of the central nervous system. Classification is based on clinical presentation, anatomical regions affected, inclusion bearing cell-types and conformationally altered proteins involved in the process. In this chapter, the current molecular pathological classification of neurodegenerative diseases...
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